| Bone-marrow cells from a patient with Bloom's syndrome cultured for 48 h in the presence of BudR exhibited a striking increase in the number of sister chromatid exchanges (SCEs) in comparison to that in the marrow cells of a patient with treated polycythemia vera (PV). Thus, it appears that an increased incidence of SCE in Bloom's syndrome occurs in various differentiated types of cells, not just blood lymphocytes, and constitutes the syndrome's most characteristic cytogenetic feature. In contrast, the incidence of SCE was not increased in marrow cells and lymphocytes of the particular PV patient studied here, whose cells did exhibit increased numbers of chromatid and chromosome gaps and breaks, presumably as result of the patient's earlier treatment. An increased frequency of SCE was demonstrated in Bloom's syndrome lymphocytes using both a technique based on BudR incorporation and one based on labeling with tritated deoxycytidine. This observation constitutes evidence against the increase of SCE being due to an unusual reaction to BudR. By conventional cytogenetic techniques, chromosome instability, including chromatid and chromosome breaks, but no homologous chromatid interchanges were also recognized in Bloom's syndrome bone-marrow cells incubated in vitro (without BudR) for either 1.k or 16 h. This observation points to the existence of chromosome instability in vivo. ( info) |
2/327. survival in a patient with polycythaemia vera for over thirty years: implications for treatment decisions in younger patients. Modern treatment has dramatically improved the prognosis for patients with polycythaemia vera (PV). Here a patient is described who has been followed and treated for PV for over 30 years, demonstrating that very long term survival can be achieved in PV. However, effective treatment modalities such as radioactive phosphorus, alkylating agents and, most recently, hydroxyurea, have all been shown to increase the risk to develop acute leukaemia when used in the treatment of myeloproliferative disorders. Hopefully, the prolonged survival of this patient may help to emphasize the need to use non-leukaemogenic agents when treating younger PV patients. ( info) |
| The tendency to evolve into acute leukemia is a well-known characteristic of polycythemia vera (PV), which is shared with the remaining chronic myeloproliferative disorders and increases after the administration of cytotoxic agents. Acute transformation is usually of myeloid phenotype, whereas acute lymphoid leukemia (ALL) following PV is seldom observed. A 63-year-old woman is described who developed ALL at 6 years from the initial diagnosis of PV, for which she had received radioactive phosphorus and hydroxyurea. The ALL was of B-cell type, corresponding to the L-3 subtype of the FAB classification. Despite the administration of combination chemotherapy the patient died shortly after the diagnosis of acute leukemia. The present case adds to seven previously described patients with the above association, all of whom had received cytotoxic therapy for PV. Median interval from PV to ALL diagnosis was 10 years, and there was a predominance of the B-cell phenotype. The prognosis was poor since all but one of the patients had a short survival after ALL diagnosis. The possible etiological and pathogenetic link between PV and the subsequent ALL is discussed. ( info) |
| True anemic infarction of the liver is a rare lesion. Most of the reported cases are due to occlusion of the hepatic artery. Whether venous occlusion can result in such anemic infarcts has been disputed although few authentic cases have been reported. A case of polycytheia vera complicated by hepatic and portal vein thrombosis with anemic infarction of a large segment of the liver is presented. The case illustrates that anemic infarcts of the liver can result from venous occlusion. The intense congestion secondary to hepatic vein occlusion may cause secondary arterial hypoperfusion unless the portal vein is available as an outflow tract. ( info) |
| The effect of renal failure and bilateral nephrectomy on erythropoiesis and plasma erythropoietic activity was observed in a patient with polycythemia vera. For eight years the patient's hematocrit was maintained between 45 and 50 per cent by phlebotomy and in spite of the development of renal failure the hematocrit did not decline. Following rejection of a renal transplant, the hematocrit fell to 18 per cent but rose to 40 per cent with oral iron therapy. Following bilateral nephrectomy, the hematocrit fell to 29 per cent but subsequently increased to 37 per cent. After an episode of gastrointestinal bleeding the hematocrit was 21 per cent but subsequently rose to 32 per cent. erythropoietin could not be detected in the plasma either before or after nephrectomy. In addition, erythropoietin failed to stimulate 59Fe incorporation into heme in vitro in the patient's marrow cells. The data incidate that, in polycythemia vera, erythropoiesis does not require erythropoietin. ( info) |
| In a 65-year-old man with polycythemia vera, invalidating angina pectoris was associated with severe narrowing of the right coronary artery. After percutaneous coronary angioplasty (PTCA) the patient became symptom free and remained so for 12 months, while receiving an antiplatelet agent, a calcium antagonist and nitrate. coronary angiography repeated after a year, because of reappearance of angina, documented good patency of the treated artery and some progression of a narrowing involving another coronary vessel. This is the first reported case of long-term success of PTCA in polycythemia vera, a disease exposed to a high risk of thrombosis and, possibly, of restenosis. It is undefined whether medical treatment contributed to the anatomical and clinical results. As far as a single case can say, polycythemia vera might not represent a prohibitive background for coronary PTCA. ( info) |
7/327. Spontaneous cervical hematoma: a report of two cases. Cervical hematomas are generally associated with trauma, surgery, and tumors. Although they are rare, they can be life-threatening because they put the patient at risk for great-vessel compression and upper airway obstruction. We describe two cases of spontaneous cervical hematoma--one in an 81-year-old man and the other in a 30-year-old woman. The man reported dysphonia, dysphagia, and neck swelling of 5 hours' duration. He had been taking 100 mg/day of aspirin for a cardiovascular condition. Examination revealed that the man had polycythemia vera. The woman was found to have neck ache, odynophagia, and cervical ecchymosis; portal hypertension, schistosomiasis, and blood dyscrasia were also found. Both patients denied trauma. A suspected diagnosis of cervical hematoma was confirmed by computed tomography, and treatment was instituted. The hematomas resolved in about 2 weeks. The treatment of cervical hematoma is controversial, although it is agreed that the evaluation of upper airway obstruction and its permeability is mandatory. Surgical treatment is generally reserved for complicated cases because of the risk of infection or bleeding. ( info) |
| A 74-year-old white man with established polycythemia vera was treated with radioactive phosphorus after phlebotomies alone failed to control his disease. About 2 3/4 years later he died of multiple myeloma. The mutagenic effect of radioactive phosphorus may have caused or possibly accelerated preexisting myeloma. Basic nonmalignant disease deserves careful consideration before radiation or radiomimetic agents are used. One might consider a probably less mutagenic drug such as hydroxyurea in patients with polycythemia vera when phlebotomy alone does not give good control of red cell mass and thrombocytosis. ( info) |
| We report here a very unusual patient with polycythemia vera treated with pipobroman who developed severe aplastic anemia following administration of the drug. Six months later, because of lack of response, cyclosporine therapy was given there was rapid and complete hematological recovery, highly suggestive of an immune-mediated mechanism, in this case. ( info) |
10/327. budd-chiari syndrome complicating restorative proctocolectomy for ulcerative colitis: report of a case. PURPOSE: This is a case of hepatic vein thrombosis presenting in a delayed fashion after proctocolectomy with ileal pouch-anal anastomosis for ulcerative colitis. Search for a causative thrombotic condition resulted in the diagnosis of polycythemia vera, a myeloproliferative disorder associated with hypercoagulability. The polycythemia was masked by an iron deficiency associated with the ulcerative colitis. methods: The history, physical, diagnostic modalities, and treatment for this patient are described, and the literature of budd-chiari syndrome associated with ulcerative colitis is reviewed. RESULTS: Six cases of budd-chiari syndrome in the setting of ulcerative colitis are reported in the literature from 1945 to 1997. CONCLUSIONS: Hepatic vein thrombosis is a rare complication of ulcerative colitis. The diagnosis of budd-chiari syndrome demands a thorough search for a hematologic condition predisposing to thrombosis. Our patient had a myeloproliferative disorder, polycythemia vera, that is associated with a hypercoagulable state. The disorder was masked by an iron deficiency associated with the ulcerative colitis. Recognition of the entity will permit successful treatment. ( info) |